Decoding pediatric spinal tumors: a single-center retrospective case series on etiology, presentation, therapeutic strategies, and outcomes.

INTRODUCTION: Spinal tumors (ST) often result in dire prognosis, carrying risks such as permanent paralysis, sensory loss, and sphincter dysfunction. Data on their incidence and etiology in pediatric populations are markedly scant. Our study investigates the etiology, clinical manifestation, treatment, and outcomes of pediatric ST. METHODS: We conducted a retrospective review of our institutional pediatric oncology and neurosurgery database, examining 14 patients under 18 years admitted with ST due to oncological diseases since 2005. We analyzed the clinical presentations, evaluations, molecular diagnostics and treatments for these patients. RESULTS: The study spanned 15 years and included 14 pediatric patients, each diagnosed with distinct spinal tumor entity. The mean patient age was approximately 19.6 ± 10.1 months. Severe axial pain along the vertebral column was observed in 13 patients, while acute neurological deterioration manifested in 7 patients. As a first-line intervention, 13 patients underwent decompressive surgery through laminectomy and tumor resection, and only one patient received chemotherapy solely. Before surgery, seven patients were unable to walk; post-surgery, six of them regained their ability to ambulate. The diagnosis encompassed a range of neoplasms: two instances of Ewing sarcoma, 3 instances of teratoma, one case presenting an atypical teratoid Rhabdoid tumor, two instances each of low-grade astrocytoma and neuroblastoma, and single instances of ependymoma, meningioma, rhabdomyosarcoma, and embryonal tumors with multilayered rosettes (ETMRs). Three patients succumbed two years after initiating therapy. CONCLUSION: Despite their rarity, intraspinal tumors in pediatric patients pose substantial therapeutic challenges. The intertwined complexities of the disease entity and the patient's neurological status demand swift initiation of an individualized therapeutic strategy. This crucial step helps optimize outcomes for this patient cohort, who frequently grapple with debilitating health conditions. Inclusion of these patients within a registry is mandatory to optimize treatment outcomes due to their rarity in pediatric population.

Autonomic dysreflexia in patients with cancer and spinal cord injury: a case series.

INTRODUCTION: Autonomic dysreflexia (AD) is a potentially life-threatening syndrome that can occur in patients with traumatic injury to the spinal cord; however, it has not been well described in patients with non-traumatic spinal cord injury (SCI) from cancer and its treatments. CASE PRESENTATION: We report four cases of autonomic dysreflexia secondary to primary spinal cord tumors and metastatic disease to the spine, and as sequela to cancer treatment. The clinical characteristics, diagnostic considerations, and therapeutic strategies used to mitigate the symptoms are discussed. DISCUSSION: The case series shows that AD is a rare but important condition to consider among patients with cancer-related SCI. There is a need for close monitoring and early identification of this syndrome in this population. Therapeutic strategies are available to mitigate these symptoms and risks of complications.

Impact of Microsurgery and Postoperative Radiotherapy on Neurological Function in Intramedullary Spinal Cord Gliomas.

OBJECTIVE: To assess the clinical efficacy of combined microsurgery and postoperative radiotherapy for the treatment of intramedullary spinal gliomas and its impact on neurological function. STUDY DESIGN: An observational study. Place and Duration of the Study: Department of Neurosurgery, Baoding No.1 Central Hospital, Hebei, China, between January 2020 and 2023. METHODOLOGY: Sixty patients diagnosed with spinal cord intramedullary gliomas were divided equally into an experimental and control group. The control group received microsurgical treatment, and the experimental group received microsurgical treatment combined with postoperative radiotherapy. The treatment effectiveness, neurological function, and follow-up results of the two groups were compared. RESULTS: After treatment, the clinical efficacy of the experimental group treatment was significantly better than that of the control group (p <0.05). The National Institutes of Health Stroke Scale (NIHSS) scores were significantly lower, and the European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-30 (EORTC QLQ-C30) scores were significantly higher in the experimental group than in the control group (p <0.05). The 1-3-year survival rate and median survival time of the experimental group were significantly higher than those of the control group (p <0.05). The incidence of complications was 3.33% in the experimental group and 6.67% in the control group, but the difference was not statistically significant (p >0.05). The postoperative recurrence rate was significantly lower in the experimental (0%) than in the control group (13.33%, p <0.05). CONCLUSION: Combined microsurgery and postoperative radiotherapy was found to be more effective than microsurgery alone. It was also more conducive to the recovery of neurological function and improved the patient's quality of life. KEY WORDS: Intramedullary spinal cord glioma, Microsurgery, Neurological function, Radiotherapy.

A 13-Year-Old Girl Affected by Melanocytic Tumors of the Central Nervous System-The Case.

Primary intracranial melanoma is a very rare brain tumor, especially when accompanied by benign intramedullary melanocytoma. Distinguishing between a primary central nervous system (CNS) lesion and metastatic melanoma is extremely difficult, especially when the primary cutaneous lesion is not visible. Here we report a 13-year-old girl admitted to the Neurosurgery Department of the Institute of Polish Mother's Health Centre in Lodz due to upper limb paresis. An intramedullary tumor of the cervical C3-C4 and an accompanying syringomyelic cavity C1-C7 were revealed. The child underwent partial removal of the tumor due to the risk of damage to spinal cord motor centers. The removed part of the tumor was diagnosed as melanocytoma. Eight months later, a neurological examination revealed paresis of the right sixth cranial nerve, accompanied by bilateral optic disc edema. Diagnostic imaging revealed a brain tumor. The girl underwent resection of both detected the tumors and an additional satellite lesion revealed during the surgery. The removed tumors were diagnosed as malignant melanomas in pathomorphological examination. Molecular analysis revealed NRASQ61K mutation in both the intracranial and the intramedullary tumor. It should be noted that in cases where available evidence is inconclusive, an integrative diagnostic process is essential to reach a definitive diagnosis.

Letter to editor: Minimally invasive tubular removal of spinal schwannoma and neurofibroma - a case series of 49 patients and review of literature.

The article on "Minimally invasive tubular removal of spinal schwannoma and neurofibroma - a case series of 49 patients and review of the literature" by Dr Argiti et al. in Neurosurgical Review journal. It is based on brief study of this article and also additional points from other references which elaborate it for further research.

Clinical evaluation of enhanced recovery versus conventional care in the perioperative period for intradural extramedullary spinal tumors: a study protocol for a multicenter, randomized controlled trial.

BACKGROUND: This randomized clinical trial protocol aimed to investigate the comparative efficacy of an enhanced recovery after surgery (ERAS) protocol versus traditional perioperative care programs in patients with intradural extramedullary spinal tumors. METHODS: The study included 180 patients aged 18-80 years, who were randomly assigned to two groups: Group A receiving traditional perioperative care and Group B receiving accelerated rehabilitation perioperative care. The nurse responsible for patient care was informed of the group assignment, but the patients themselves remained blinded to the intervention. The primary outcome measure was the Karnofsky Performance Scale score, which assessed functional status. The secondary outcomes included the Japanese Orthopedic Association Scale, Numeric Pain Rating Scale, length of postoperative hospital stay, duration of urethral catheterization, patient satisfaction questionnaire, and complication rates. Follow-up assessments were conducted telephonically 1 month, 3 months, and 6 months after the surgery. DISCUSSION: This study protocol provided a structured approach to assess the potential benefits of ERAS during the perioperative period for patients with intradural extramedullary tumors, aiming to improve patient outcomes and overall care efficiency. TRIAL REGISTRATION: This study has been registered with the China Clinical Trials Registry (Project No: ChiCTR2200063347). Registered on September 5 2022.

Retinol dehydrogenase 10 promotes epithelial-mesenchymal transition in spinal cord gliomas via PI3K/AKT pathway.

Background: Spinal cord glioma (SCG), a rare subset of central nervous system (CNS) glioma, represents a complex challenge in neuro-oncology. There has been research showing that Retinol Dehydrogenase 10 (RDH10) may be a tumor promoting factor in brain glioma, but the biological effects of RDH10 remain undefined in SCG. Methods: We performed gene set enrichment analysis (GSEA) and unsupervised clustering analysis to investigate the roles of EMT (epithelial-mesenchymal transition) in glioma. DEG (differently expressed gene) screening and correlation analysis were conducted to filter the candidate genes which were closely associated with EMT process in SCG. Enrichment analysis and GSVA (Gene Set Variation Analysis) were conducted to investigate the potential mechanism of RDH10 for SCG. Trans-well and healing assay were performed to explore the role of RDH10 in the invasion of SCG. Western blotting was performed to evaluate the levels of markers in PI3K-AKT and EMT pathway. In vivo tests were conducted to verify the role of RDH10 in EMT process. Results: Bioinformatic analysis demonstrated the EMT pathway was associated with dismal prognosis of glioma. Further analysis demonstrated that RDH10 showed the strongest correlation with the EMT process. Retinol Dehydrogenase 10 expression was significantly increased in SCG tissues, correlating with advanced tumor grade and unfavorable prognosis. Functional analysis indicated that decreasing RDH10 levels impeded the invasive and migratory abilities of SCG cells, whereas increasing RDH10 levels augmented them. Enrichment analysis and western blot revealed that RDH10 regulated EMT process of SCG by PI3K-AKT pathway. We observed that the enhanced invasion ability and increased EMT-related protein induced by RDH10 overexpression can be suppressed by PI3K-AKT pathway inhibitor (LY294002). Conclusion: Our research found that RDH10 was an effective biomarker associated with tumor grade and prognosis of SCG. RDH10 could regulate EMT process of SCG through PI3K-AKT pathway.

Understanding spinal cord astrocytoma: Molecular mechanism, therapy, and comprehensive management.

Spinal cord astrocytoma is a rare and highly debilitating tumor, yet our knowledge of its clinical characteristics, molecular features, and pathogenesis remains limited compared to that of its counterparts in the brain. Current diagnostic and therapeutic approaches for spinal cord astrocytomas are primarily based on established guidelines for brain astrocytomas. However, recent studies have revealed unique clinical and pathological attributes that distinguish spinal cord astrocytomas from their corresponding brain counterparts. These findings underscore the inadequacy of directly applying the clinical guidelines developed for brain astrocytomas to spinal astrocytomas. In this review, we provided an up-to-date overview of the advancements in understanding spinal cord astrocytomas. We also discussed the challenges and future research prospects in this field with the aim of improving the precision of diagnosis and therapy for these tumors. Specifically, we emphasized the importance of enhancing our understanding of the molecular heterogeneity, immune characteristics, and clinical trials of spinal cord astrocytomas.

Vascular Pathologic Conditions in and around the Spinal Cord.

Diagnosing and differentiating spinal vascular pathologic conditions is challenging. Small structures, lengthy imaging examinations, and overlapping imaging features increase the difficulty. Yet, subtle findings and helpful protocols can narrow the differential diagnosis. The authors aim to help radiologists make accurate and timely diagnoses of spinal vascular pathologic conditions in and around the spinal cord by highlighting spinal vascular anatomy, imaging findings, and three broad categories of abnormalities: infarcts, anomalies, and tumors. ©RSNA, 2024.

A Concise Guide to D-Wave Monitoring during Intramedullary Spinal Cord Tumour Surgery.

D-waves (also called direct waves) result from the direct activation of fast-conducting, thickly myelinated corticospinal tract (CST) fibres after a single electrical stimulus. During intraoperative neurophysiological monitoring, D-waves are used to assess the long-term motor outcomes of patients undergoing surgery for intramedullary spinal cord tumours, selected cases of intradural extramedullary tumours and surgery for syringomyelia. In the present manuscript, we discuss D-wave monitoring and its role as a tool for monitoring the CST during spinal cord surgery. We describe the neurophysiological background and provide some recommendations for recording and stimulation, as well as possible future perspectives. Further, we introduce the concept of anti D-wave and present an illustrative case with successful recordings.

[Stereotactic high-dose irradiation for intramedullary metastases]. / Stereotaksicheskoe vysokodoznoe obluchenie v lechenii intramedullyarnykh metastazov.

Intramedullary metastases (IMM) lead to severe neurological symptoms. They are extremely rare in clinical practice. Optimal management of such patients is not clearly defined. Surgery, chemo- and radiotherapy are the main options. OBJECTIVE: To study the results of stereotactic high-dose irradiation for IMM considering local control of metastases, neurological status and life expectancy. MATERIAL AND METHODS: Nineteen patients with 29 IMMs underwent high-dose irradiation (Cyber Knife G4 and TrueBeam STx devices) between 2016 and 2022. There were 1-10 fractions, radiation dose 14.0-35.0 Gy with isodose 70-100%. Local control and new spinal cord metastases were assessed using contrast-enhanced MRI. Clinical status and neurological functions were assessed using the Karnofsky and McCormick scales. RESULTS: Local control rates were 91% and 78% after 6 and 12 months, respectively. Overall survival after treatment was 7.3 months, 6- and 12-month overall survival - 58% and 37%, respectively. In addition, 8 patients were available for follow-up. Of these, 6 ones demonstrated improvement or stabilization of neurological function after treatment. The main cause of death was progression of the underlying disease. No significant treatment-related toxic effects were observed. CONCLUSION: High-dose irradiation is a safe, effective and time-saving treatment for IMM. Considering unadvisable surgical methods and chemotherapy for this pathology, we can recommend high-dose irradiation for patients with spinal cord metastases.

Minimally invasive tubular removal of spinal schwannoma and neurofibroma - a case series of 49 patients and review of the literature.

To evaluate the efficacy and safety of minimally invasive tubular removal of spinal schwannoma and neurofibroma. In this single-centre study, we retrospectively analysed 49 consecutive patients who underwent minimally invasive removal of a total of 51 benign spinal nerve sheath tumors using a non-expandable (n = 18) or expandable tubular retractor (n = 33) retractor system between June 2007 and December 2019. The extent of resection, surgical complications, neurological outcome, operative time, and estimated blood loss were recorded. Histopathology revealed 41 schwannomas and 10 neurofibromas. After a mean follow-up of 30.8 months, postoperative MRI showed gross total resection in 93.7%, and subtotal resection in 6.3% of the tumors. Three patients were lost to follow up. Of the subtotal resections, one was a schwannoma (2.4% subtotal resections in schwannomas) and two were neurofibromas (20.0% subtotal resections in neurofibromas). Intraspinal and paraspinal tumor localizations were equally accessible by minimally invasive tubular surgery. Conversion to open surgery was not required in any case. The mean operative time was 167 ± 68 min, and estimated blood loss was 138 ± 145 ml. We observed no major surgical complications. Spinal schwannoma and neurofibroma can be removed effectively and safely using a minimally invasive tubular approach, with satisfying extent of tumor resection comparable to the conventional open surgical technique and no increased risk for neurological deterioration.

Epidemiological features of spinal intradural tumors, a single-center clinical study in Beijing, China.

BACKGROUND: Spinal intradural tumors are rare and heterogeneous in histological type, aggressiveness, and symptomatology, and there is a lack of data about them. This study investigated the epidemiological features of spinal intradural tumors. METHODS: This retrospective analysis included patients with spinal intradural tumors who underwent surgical treatment at the Myelopathy and Spondylosis Ward Beijing Jishuitan Hospital between January 2012 and December 2022. RESULTS: This study included 1321 patients [aged 47.19 ± 14.90 years, 603 (45.65%) males] with spinal intradural tumors. The most common histological subtype was schwannoma [n = 511 (38.68%)], followed by spinal meningioma [n = 184 (13.93%)] and ependymoma [n = 101 (7.65%)]. Fifteen (1.14%) patients were diagnosed with metastatic spinal intradural tumors as a presentation of another primary cancer type. The spinal intradural tumors were mostly found in the lumbar region [n = 436 (33.01%)], followed by the thoracic vertebrae [n = 390 (29.52%)], cervical vertebrae [n = 154 (11.66%)], and thoracolumbar region [n = 111 (8.40%)]. Schwannomas mostly affected the lumbar region [n = 256 (52.64%)], spinal meningiomas in the thoracic region [n = 153 (83.15)], and ependymomas in the lumbar region [56 (55.45%)]. The de novo metastases were mostly found in the lumbar region [n = 8 (53.33%)]. CONCLUSION: According to the results of our single-center study, the most common spinal intradural tumor in Northern China is schwannoma, followed by spinal meningioma and ependymoma.

Does Minimally Invasive Hemilaminectomy for Intradural Extramedullary Tumor Resection Prevent Postlaminectomy Kyphosis or Lordosis Loss?

BACKGROUND: Minimally invasive hemilaminectomy is a safe and effective alternative to open laminectomy for treating intradural extramedullary tumors. There are no reports of postoperative kyphosis after this approach. This study aims to determine whether performing minimally invasive spine surgery hemilaminectomy for intradural extramedullary tumors can prevent the development of postlaminectomy kyphosis (PLK) or lordosis loss. MATERIAL AND METHODS: Sixty-five patients with spinal intradural extramedullary tumors who underwent minimally invasive hemilaminectomy surgery and complete pre and postoperative radiologic imaging were included. The effect of the surgical approach on the spinal sagittal axis was assessed by comparing pre- versus postoperative segmental and local Cobb angles at different spinal levels, considering anatomical localization (cervical, thoracic, lumbar, and transition segments) and functional features (mobile, semi-rigid, and transition segments), as well as the extent of the surgical approach (1, 2, or 3 levels) and follow-up. RESULTS: None of the patients had an increase in thoracic kyphosis nor a loss of cervical or lumbar lordosis greater than or equal to 10° after undergoing the minimally invasive spine surgery hemilaminectomy approach. More than 5° of increase in kyphosis was detected on 7.4% and 11.1%, for the segmental and the local angles, respectively; meanwhile, for patients with loss of lordosis, this deviation was detected in 5.3%, for both angles. The occurrence of PLK was more common than that of lordosis loss, but mainly manifested in postoperative angle impairment of less than 5°. No significant differences were evidenced, considering the approach length. CONCLUSIONS: Hemilaminectomy represents a promising approach for preventing PLK and postlaminectomy lordosis loss following intradural extramedullary tumor resection.

Predictors of Functional Independence in Children with Spinal Cord Tumors After Surgery: From Physiotherapist Perspective.

Knowledge of the factors affecting functional outcomes has an important role in the development of the care plan in children with spinal cord tumors. Retrospective study was planned to determine predictors of functional independence in children with spinal cord tumors. Data from 80 children treated for spinal cord tumors over 2001-2020-year period was analyzed. Results showed that key predictors such as younger age, higher KPS, and better initial WeeFIM scores were strongly associated with improved functional outcomes. Further multicenter prospective studies are recommended to validate these findings and explore long-term functional outcomes to enhance rehabilitation strategies for this patient population.

The utility of intraoperative ultrasonography for spinal cord surgery.

OBJECTIVES: Intraoperative ultrasonography (IOUS) offers the advantage of providing real-time imaging features, yet it is not generally used. This study aims to discuss the benefits of utilizing IOUS in spinal cord surgery and review related literature. MATERIALS AND METHODS: Patients who underwent spinal cord surgery utilizing IOUS at a single institution were retrospectively collected and analyzed to evaluate the benefits derived from the use of IOUS. RESULTS: A total of 43 consecutive patients were analyzed. Schwannoma was the most common tumor (35%), followed by cavernous angioma (23%) and ependymoma (16%). IOUS confirmed tumor extent and location before dura opening in 42 patients (97.7%). It was particularly helpful for myelotomy in deep-seated intramedullary lesions to minimize neural injury in 13 patients (31.0% of 42 patients). IOUS also detected residual or hidden lesions in 3 patients (7.0%) and verified the absence of hematoma post-tumor removal in 23 patients (53.5%). In 3 patients (7.0%), confirming no intradural lesions after removing extradural tumors avoided additional dural incisions. IOUS identified surrounding blood vessels and detected dural defects in one patient (2.3%) respectively. CONCLUSIONS: The IOUS can be a valuable tool for spinal cord surgery in identifying the exact location of the pathologic lesions, confirming the completeness of surgery, and minimizing the risk of neural and vascular injury in a real-time fashion.

Diffuse lymphoma involvement of the spinal cord showed on [18F]FDG PET/MRI.

A 61-year-old woman with diffuse large B-cell lymphoma received a fluorine-18-deoxyglucose positron emission tomography/computed tomography ([¹8F]FDG PET/CT) for staging. Because of the obvious uptake of [¹8F]FDG in the spinal cord and brain, a positron emission tomography/magnetic resonance imaging (PET/MRI) was performed after the positron emission tomography/computed tomography (PET/CT). The images showed diffuse [¹8F]FDG uptake of the spinal cord and increased T2 signal intensity on MRI, which was suspected to be lymphoma involvement. The patient was diagnosed with diffuse large B-cell lymphoma involving the right maxillofacial region, right cervical lymph nodes, cervix, brain and spinal cord (stage IV of non-germinal center B-cell origin). After chemotherapy, the spinal [¹8F]FDG uptake level decreased significantly, which was considered to be a partial metabolic response. Our case was different from prior, which indicated the pattern of spinal cord involvement by lymphoma was focal.